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A 53-Year-Old Woman with Proximal Limb Weakness and Marked CK Elevation

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Abstract

Immune-mediated necrotizing myopathy is a type of idiopathic inflammatory myopathy. It is characterized by subacute, progressive, symmetric, and proximal limb weakness, marked serum creatine kinase (CK) elevation, myofiber necrosis on muscle biopsy with diffuse or multifocal myofiber upregulation of class I major histocompatibility complex (MHC1) but no or minimal interstitial lymphocytic infiltrates, and association with anti-3-hydroxy-3-methyglutaryl-coenzyme A reductase (HMGCR) or anti-signal recognition particle (SRP) autoantibody in a subset of patients. Here we present a case of immune-mediated necrotizing myopathy associated with anti-SRP autoantibody, which is severe and refractory to immunosuppressive therapy.

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Author information

Authors and Affiliations

  1. Departments of Neurology and Pathology, Boston University Medical Center, Boston, MA, USA

    Lan Zhou

  2. Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Chunyu Cai

Authors
  1. Lan Zhou
  2. Chunyu Cai

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Correspondence toLan Zhou.

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Editors and Affiliations

  1. Departments of Neurology and Pathology, Boston University Medical Center, Boston, MA, USA

    Lan Zhou

  2. Department of Pathology, Neuropathology Section, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Dennis K. Burns

  3. Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Chunyu Cai

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Zhou, L., Cai, C. (2020). A 53-Year-Old Woman with Proximal Limb Weakness and Marked CK Elevation. In: Zhou, L., Burns, D., Cai, C. (eds) A Case-Based Guide to Neuromuscular Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-25682-1_7

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